Written by Clay Smith
See if you can crack this case of pediatric syncope. It took suspicion and persistence to get the diagnosis. Would you have made it?
Why does this matter?
Pediatric syncope is uncommon and usually benign…except when it’s not. Test yourself with this case so you won’t miss this challenging diagnosis in the future.
Mystery syncope case
This is a 9 year old girl with four prior episodes of syncope, some with a few tonic jerks; all with ashen appearance, not associated with fever or exertion. Her workup with EEG, head CT, neurology and GI consults was all negative. She also had a negative echo, ECG, and Holter. The father had a diagnosis of vasovagal syncope and had a normal appearing ECG at the time of her presentation. Due to her recurrent, unexplained episodes and father’s history of syncope, an implantable loop recorder was placed. She had hundreds of episodes of dizziness reported, all of which showed normal sinus rhythm on review of the tracings. Twenty months later, however, she had fever, lightheadedness, and a transient wide complex tachycardia (WCT) recorded. But subsequent ECG, echo, exercise test, procainamide challenge, and other EP tests were all negative. Here is her baseline afebrile ECG.
Finally, more testing produced v-tach/v-fib in the EP lab, and an ICD was considered. Yet, her ECG always looked normal. But then…she had another febrile illness. Of note, the father also had an abnormal ECG during a febrile episode in the past. Her ECG looked like this with fever. See if you can make the diagnosis before you scroll past the ECG for the discussion.
This is diagnostic for Brugada sign.
Here are the key points on Brugada syndrome you need to know.
It is autosomal dominant, usually seen in older males (not children), and more often seen in Asian people.
It is characterized by the downsloping ST-elevations in V1-3, but this may be intermittent and only seen with fever.
Procainamide challenge may be falsely negative in children.
Brugada syndrome may be responsible for 4 to 12% of sudden cardiac death in children and young athletes.
Placing an ICD in a child is a tough call, with good arguments for and against. Ultimately, this child got one.
If you see a febrile patient with Brugada, or suspected Brugada, treat fever aggressively with antipyretics.
If you happen to be caring for a child with recurrent, unexplained syncope who is febrile, you may want to get an ECG with fever. This could help make the diagnosis.
For an in depth analysis of the ECG morphologies in Brugada types 1 and 2, you need to read Dr. Smith’s ECG blog post. It is gold.
Suspicion and Persistence: A Case of…. Pediatrics. 2019 Jul;144(1). pii: e20183296. doi: 10.1542/peds.2018-3296. Epub 2019 Jun 12.
Open in Read by QxMD
Reviewed by Thomas Davis